Tuesday, 31 July 2012

This surgery scared the heebie jeebies out of me though I put on a very brave front. This was a massive deal for a 9 year old girl.

Dr Emmett explained what he was going to do to me, step by step, using a real human skull. That in itself gave me more heebie jeebies. He had told me that it had come from India. That was a real alive human once and now it was his model. That skull will forever be imprinted in my mind.

All preparations were complete, the operation planned and the surgeons went to the University of Queensland to practise the
operation beforehand.

The Queensland Craniofacial Clinic was launched with the surgery on a young boy called Robert Hoge. It was big news around the country. I have the clipping from the front page of The Australian.

This is Robert today.

I came next - no silver medal. Just the team's report in a medical journal that says that I was the first patient operated on in Queensland with Crouzon Syndrome. That's my claim to fame.

Me

In a marathon 10 hour
operation the surgeons firstly cut my head open from ear to ear to access my face. They then advanced my top jaw 1.5 cm to approximate with my lower
jaw and held it there with rib bone blocks. They also moved my eye sockets
forward and held them forward with a bar of bone above and blocks of bone below.
Hip and rib were used. The bone was crafted to shape and fixed in position by
stainless steel wire. My jaws were wired together with tooth caps and splints. I had a tracheotomy so I could breathe.

My parents waited in the hospital pacing the floor...waiting, wondering. A lady from church came and held Mum's hand and prayed for me throughout the operation. My mum appreciated her gesture of kindess and support. It was a very long wait.

Of course with all operations there are risks. You are always told the risks before surgery and usually cringe at the long list. You also hope and pray that complications don't happen and that there will be a perfect outcome. But no, that was not the case for me.

What Went Wrong?In ICU the surgeons noticed that my left eye was
more swollen and prominent then my right eye. They thought that maybe there was a misplaced block of bone in the eye socket so they took me back into theatre again to have a look. But no there wasn't...my left eye just had a massive amount of swelling. The severe swelling led to damage of my optic nerve and very low vision resulted. I am legally blind in that eye. Muscular stretching around that eye also was
put down to having happened in the operation. Nothing could be done to correct these.

Dr Spiro had been correct in his earlier warning.

With these complications my
parents questioned themselves as to whether they had made the right
decision but you can't go back in time. What was done, was done. You can only move on.

MemoriesI have vivid memories of my stay in the hospital and the operation. I remember:

crying and feeling all alone on the first day after Mum and Dad left. I didn't like being in hospital all on my own. It was really scary.

being in a bed that was like a big kid's cot which I couldn't get out of. Rows of them in one big room.

being given lots of new books and colouring pencils to keep me busy in bed.

going to the hospital school before the operation - it was a wonderful novelty.

after the operation being
in ICU and not being able to talk or see. My eyes were bandaged and my jaws were wired together. I would try to write on a notepad or point if I wanted something.

my Dad's
transistor radio kept the loneliness away. When I hear the songs of the day particularly
'Leo' and 'Video Killed the Radio Star' I am taken back to that bed.

my Dad reading Magpie Island
by Colin Thiele to me.

the painful physio I had on my chest, painful because I had just had ribs cut out.

back in the ward re-learning how to walk. I had lost the ability from being in bed for so long.

having all my meals as a soup as my jaws were wired shut.

not having any hair as it was shaved off and the nurses trying to make me wear one of Robert's beanies which I didn't want to wear. I didn't want to wear anything - I didn't care that I had an ear to ear scar across my head. But I guess others didn't want to see it so Mum bought me some beautiful silk scarves to wear. And once we got out of hospital Mum bought me a wig to wear.

having my friend Anne from school come and visit me. Also seeing my cousins. My cousins brought me an Ekka bag as it was Ekka time and one of the nurses brought me a scally wag doll.

receiving letters from my classmates.

being given a picture of Jesus by one of the nuns (which I still have).

handing out lollies to all the patients in the ward but not initially being able to eat them myself.

the hospital throwing me a surprise 10th birthday party in the ward but because I could not eat any of it, I sulked on my bed.

having the splints taken off my teeth (which I
still have) which was painful as I was only lighted sedated.

finally going home.

At school wearing my wig, still with swollen eyes

A year later

After I went home life continued. I became shy and did not want to go out. I did not want to go back to Brownies or school but my mum pushed me.

Kids wearing wigs would have been a very uncommon sight in the 1970s. And uncommon sights meant more teasing.

And of course that's what happened to me. I had just gone through all the trauma of the operation and then was subjected to more bullying once I got back to school. One strong memory was at the school swimming carnival. I was swimming in a race so I had to take the wig off to put on my cap. Afterwards I was coming out of the toilet block and was readjusting the wig, and a girl saw me and brought all her friends to tease me about it. I said I wasn't wearing a wig and my friends told them to go away. I was kicking myself for readjusting it outside of the change room.

I also have a memory of the boys in my class saying that I was wearing a wig and I kept on denying it - guess I desperately wanted it to be my own hair...Then when I finally took the wig off when my hair had grown a little, they knew they were right..but I still wouldn't admit that I had worn a wig.

Looking at the school photo above, it was soooo obvious.

Once all the swelling came down my face looked great and I was happy. And of course my precious hair grew back.

On the Christmas holidays at a beach mission, I gave my life to Jesus.

Jumping the fence, off I went to primary school. The kindy was next door to the primary school and we would often peer through the mesh in the dividing fence and look at the children who were playing happily and noisily on the swings and around the giant old gnarled trees that grew in the grounds. Now I was there.

Do you think it is in primary school that your personality is formed? Your self-esteem is built or torn down? Your self-worth as perceived by others around you are nurtured or destroyed? I do.

Roses, camellias, daisies, African violets = size of popularity

Primary school was reflective of the world outside the gates that I had been introduced to as a toddler. There were the kind protective friends who I had made in kindy and others I had made at school. The children who came from kindy knew me 'as me' with no judgement and they loved me for who I was. Lunchtimes were filled with playing on the playground, beam, hopscotch, elastics, knuckles, playing with our Barbies or writing stories. My friends invited me to parties, sleepovers, and on play dates outside of school too. Being with my friends equaled fun and good times.

But there were also the self-esteem destroying tongues which tortured me with their name calling. Majority being boys. I can still hear their voices in my head. Ignoring them and telling these monsters to go away didn't stop them from targeting me. Sometimes the verbal bullying became physical with me being pushed over. I never fought back.

No anti-bullying campaigns, no adults standing up for you, not even my mum who became a teacher at the school when I was in Year 2, could stop the name calling...no discipline for name callers. Your friends and your siblings were your anti-bullying campaign...and they were for me.

I loved learning and did well academically.

Outside of school I was involved in Brownies and then went onto Girl Guides. I loved both. I also went to tennis and swimming lessons and learnt to ride on my mother's adult bike.

I also loved it when my cousins came to visit my Nana and Pop who lived next door to us, or my other grandparents who lived in the next street. My cousins knew me 'as me' too and we just played and had heaps of fun. Playing dress ups, singing, dancing, laughing, playing games, playing with water etc. are all wonderful childhood memories I have.

In those early years I continued to be monitored by the paediatrician. Apart from needing glasses, which I refused to wear, I was fine. Wearing glasses invited more teasing and I wasn't going to open myself to more.

In 1975 Dr Stable referred me to an oral surgeon and a plastic surgeon to see if they could help me. They did not have the expertise to help me but they told my parents that the Australian Craniofacial Clinic had opened in Adelaide led by Professor David David and that was an available option.

Could there be hope for my face? Should they take me there?

While my parents were contemplating whether to take me to Adelaide there came news that a craniofacial clinic was being set up in my state. My parents were surprised. The following year in 1976, I went to see Dr Tony Emmett, the plastic and reconstructive
surgeon. Dr Emmett had spent two years in Paris learning the revolutionary reconstructive
techniques from Dr Paul Tessier who had pioneered craniofacial surgery and knew Crouzons.

Dr
Emmett told my parents that there was surgery available that could correct and rebuild my facial
features. Mum and Dad were nervously excited.

Over the ensuing months, I went to see various specialists and had
x-rays and photos taken. I was to have a plaster mould of my head taken (with straws up my nostrils) but thankfully they changed their mind. I didn't want to have that done!

But not all my specialists were supportive of me having the surgery. My eye specialist Dr Paul Spiro
was adamant that I should not have the surgery as it was too great of a risk to my eyesight.

What would you do?

My parents weighed everything up and decided to go with it hoping and praying for the
best.

Monday, 30 July 2012

Our dream is for Jenny to grow up to be healthy and an independent member of society.

This was my parent's desire for me.

So even though I had Crouzons, my parents set about bringing me up and disciplining me like any other regular child. I always wore nice handmade clothes and my hair was nicely brushed and put up, perfected to hide the scars on my head. My mum and nana were great at sewing, knitting and crocheting and they made me matching outfits for my Barbie doll and my big dolls. I was loved and well cared for.

To achieve the goal of being independent later on in life, I was taken out into the world. I was not hidden away in the house....as may children of that era were if they had disabilities. I guess they were trail blazers in that regard. Pioneer advocates and I am thankful to them for that.

Church, family and friends were very supportive of my parents. There being no support group they made their own. Friends who had a daughter with hydrocephalus became heart friends. They suppoorted each other through the tough times. I remember visiting them a lot growing up and seeing the little girl get very sick and pass away.

Physical fights were a common occurrence for my brothers. Kids can be very cruel and my brothers were very protective of me. They both have psychological scars today from growing up having me as their sister.

The local adults who were used to seeing me around took no notice. My nana would hear cruel comments but they were never said to my mum in person. When my parents took me away from my town that is where they encountered 'in your face' ignorance, cruel comments and stares. They needed to have thick skins to deal with what they went through. Mum has told me the story that apparently when people would stare at me for a long time, that I would open my eyes up really big and stare back until they looked away! Feisty girl. My parents also had to be forceful to get the same service as other people got. There was an incident at a puppet show where the photographer refused to take my photo with my brothers. Mum demanded he take it. He relented but he refused to pose us and it was taken very quickly.

Determined that I would have as normal a childhood as possible. off I went to kindergarten. It was advantageous for me having my nana and mum working there. I got to have the birthday jelly babies after school! Kindy was fun and I have only good memories - the wooden school building, the beds, the slides, the playing, the sandpit, the free milk, the Christmas play. So long ago but still so clear.

It was a haven from the sometimes nasty world outside.

And I made my first friends there...some I still have today.

The other place I loved going to, was Sunday School. Dressed in my Sunday best pretty dresses and going into the Minister's house for lessons. I loved singing around the piano. 'Jesus loves me yes I know for the bible tells me so...', collecting little cards with verses on them, and the cordial and biscuits at the end. Jesus sowed his seed in my heart at this early age.

Mum and Dad dressed me in a hand made frilly dress, a bonnet and booties, wrapped me in a delicate pink shawl and took me home. This was a time of joy but also heartache. My parents had to come to terms with how their baby looked, not knowing whether I would live or die. More questions but no answers...... would I look like this always? Was I intellectually normal? Many many questions swirling, erupting. No one around who had been in their shoes. Loneliness can be cruel.

On top of coming to terms with me, and going through the mourning process of not having the baby they thought they were going to have.....they had to endure what the community had to say about my face and the constant staring. This extended to my brothers too who had to contend with the children's opinions of their little sister. The good, the bad and the ugly comments.

Though always a rose in my Nana's heart.

My family's life would never the same. There would be joy, tears, laughter, physical and emotional scars and love.

I was referred to the paediatrician, Dr Grantley Stable in Brisbane. He was the one who diagnosed me. He would have looked through his thick medical textbook until he found a photo of someone that looked something like me. Then he would have read the characteristics and decided if they matched me. If they did, then that was what I had. Today genetic testing gives the diagnosis. Dr Stable decided that I had Crouzon Disease (now called a syndrome). This diagnosis came to be questioned when my first daughter was born.

Crouzon Disease/Syndrome (also known as Craniofacial Dysostosis) was
originally described in 1912 by O. Crouzon in Paris in a mother and her
daughter. It is caused by a mutation in Chromosome 10 n the FGFR2 (fibroblast growth factor
receptor-2) gene or in the FGFR3 gene (known as acanthosis nigricans when a dark
discolouration occurs with rough skin in the armpits and groin). This mutation means that the cells that form the skull and face stop growing too early causing small bones.

I went to see Dr Stable at 3 months, 6 months and 12 months. He was an angel in Mum and Dad's world offering them kind support. An ear to listen, who they could be honest with. Something they needed emotionally and psychologically. At each visit the usual checks of weight, height, reactions, responses, feeding etc. were done and all were normal. I met all milestones e.g. smiling, crawling and standing up in my cot.

9 months old

As I started to grow my facial syndrome became more pronounced. I was sent to have x-rays at 12 months. Mum remembers vividly how she had to hold my head still for the x-rays but I was not co-operative. Mum got upset and Dad took over. The radiologist was unhappy that he had to re-explain to Dad what to do. Today the baby would be sedated....

The x-rays showed that my brain was pressing on the skull. An operation had to be performed quickly or mental impairment and eventually death would occur.

But there lay the problem. This was the late 1960s. No one had seen this before so who could help me? I can only imagine how scared my parents felt.

There was also divided opinion on this surgery.

Dr Stable wanted to save my life. He was determined to find a surgeon to help me.
On the other hand the Professors at the University of Queensland said it would be a waste of time so not to do it....let me die....

Reach for the hope of life...or do nothing and allow death to occur....

An easy choice for my parents.

Dr Stable was told to find the surgeon. They were going to take the risk. There really was nothing to lose. This was their baby's life. This was love.

Dr Stable was the angel who found the surgeon to help me.

I was referred to Dr Ken Jamieson, also in Brisbane. He was a neurosurgeon who was renown for rebuilding car accident victims' skulls. Dr Jamieson had never encountered craniosynostosis (fusion of the skull bones) before but was prepared to help me.

Dr Jamieson is the reason I am alive today.

A month later I was in hospital. The operations were done in two stages - one half of the skull at a time.

Dr Jamieson dissected the skull into flaps of bone and implanted plastic between the sections to stop the bone from growing back together. Both operations were to be completed within three weeks but the second operation was postponed for a week as I had an infection. I was in the hospital for 6 weeks, not the three as planned.

Hospitals in the 1960s were not parent friendly like today. There was no staying overnight, strict rules, strict visiting hours, strict nurses. No counselling, no social workers.

Seeing me after my first operation was a shock to my parents. My black eyes and head bandages were too much for them to cope with. My crying when they went home still rings in my mother's heart.

As well as emotionally draining my operations were financially draining as the hospital would not reduce its fees for my parents even though I was staying twice as long as originally planned and they had medical insurance. Strict rules, no compassion.

After my bandages came off the healing began and my development continued. It was after these operations that I walked independently.

The doctors concluded that these operations were a success but that nothing more could be done for me as at that time no facial construction surgery existed.

My story begins in a small country town. A town with no traffic lights, one main street through the middle, a line of shops, and where everyone knew everyone and would stop to have a chat... My parents and their families were well known and respected in the town. My mother was a high school teacher and my father a plumber.

My parents had two beautiful boys. One dark haired and the other a blonde. Both though different in personalities, were a joy. Then a surprise - another pregnancy. Preparations, excitement...would it be a boy or a girl?

There were no scans or other types of tests in the 1960s so as far as anyone knew this pregnancy was going to produce another perfect baby...just like my two brothers.

Oh how wrong they were. How shocked was everyone going to be when I appeared in the world?

My Birth Day....
I was two weeks overdue and my mother was induced. Dad was at work - fathers weren't involved in the birthing process in the '60s.

Lots of babies have to be induced so nothing untoward there. It was a hard labour for my mother though again nothing unusual. I arrived whilst the obstretician was performing a caesearan on another patient so I was delivered by a mid-wife. Mum saw me briefly and I was whisked away. No one came near Mum and she didn't know what was going on. She was confused. What was wrong with her baby?

The next indicator to Mum that there was a major problem with me was when she next overheard the nurse on the phone. She had rung Dad and told him to come to the hospital straight away as there was a problem with the baby.

When Dad arrived they were told together that I had some type of craniofacial deformity. Now this was not a missing arm, or deafness or even hip dysplasia. This was in your face...my face...a small skull, bulgy eyes, large lower jaw. Not normal. Very hard to hide or pretend nothing is wrong.

I feel for Mum and Dad. There were no social workers, no counsellors, no support groups or social media. They faced 'my face' alone. Just with numbness and shock as their friend. I can only imagine the devastation and the anger and the questions - what went wrong? Why did this happen?

That afternoon my Nana came to the hospital to see me. She told Mum that I was beautiful. Through tears Mum said that no I didn't look beautiful. My mum's world had fallen apart and she was full of a mixture of emotions, from fear to guilt. But through all these mixed emotions there was no thought of giving me up for adoption for allowing me to die by starvation.

Later that same day the paediatician also arrived and told Mum and Dad that the bad news was that my skull was fully fused (called craniosynostosis) - I had no soft spots which meant I would probably end up mentally impaired and dying. But the good news was that I could see! Ha! More shock...

I had reflux and was kept in the hospital for 2 weeks until I was feeding properly. Mum came down with a cold sore from all the stress and was not allowed to go near me, so she went home and left me at the hospital until it was time for me to go home. I was kept in the nursery with the babies who were up for adoption only having contact with the nurses and the doctor.

First Question...
Who decided that a rose is a beautiful flower and should be attributed such prestige and to love? Do you like roses? Not everyone does. I prefer gerberas and had them in my wedding bouquet, no rose to be seen. And I love camellias. They're as pretty as roses. Camellias mean love to me as they were growing in my Nana's garden and I loved her dearly. My mother loves chrysanthemums. Maybe you like daisies or carnations or African violets.

Second Question and Third Question...
Is a ham sandwich better than a cheese sandwich? Is broccoli better than cauliflower?

Still more questions...
Who decided what a beautiful face looks like? Is it a face with high cheekbones, dimples, a heart shaped face, smooth skin, pumped lips, a botoxed forehead? Scientists have identified a formula for calculating the most 'beautiful face'. If you haven't heard of this click below:

According to the formula, Jessica Alba, Liz Hurley and Shania Twain are perfectly beautiful. Do you agree?

Just like the type of flower you like, the type of sandwich, the vegetable, or who you think is beautiful.... it is an individual opinion. Is there a right or wrong answer? Probably not but there are people who will adamantly say that one is better than the other and put up all sorts of reasons for their justification.

When you are born with a facial difference or a syndrome such as Downs, people's perceptions of how beautiful you are are forced upon you. How you are treated, the stares people give or the avoidance, the bullying, or even the help and support, all tell you what they think of how you look and the value they place on your life. Strangers particularly will form an opinion of you without even knowing you. Friends and family who know you will usually have a completely different opinion of you...is a rose better than a camellia?

After so many questions and jabbering on....welcome to my blog. This is the story of my journey. And yes I was born with a face that was different... and I have children with a facial difference and I have a child with Down syndrome. Have you already formed an opinion of me?

This is a brief overview of what my blog will be talking about:

Iwas born in the 1960s in a small country town in Australia,. I was born with Crouzon syndrome - a rare craniofacial syndrome. I grew up in a time where disabilities or differences were not tolerated very well. These children were often hidden away from society.My life was saved as a baby.I was bullied often at primary and high school and regularly felt rejected because of how my face looked. I underwent pioneering reconstructive facial surgeries in an attempt to fit into society's standard of what is acceptable for a face to look like.After high school I studied to become a primary school teacher. My first 13 year relationship was verbally and emotionally abusive. I followed what I had always known. Eventually with great courage, I left. I married a kind, loving man who I found on the Internet. We have three beautiful children - two have Crouzon syndrome and my youngest has Down syndrome. My children have had a lot of operations and therapies. The local hospital is our second home.

My blog will also be showing how my faith in God has helped me to find joy in my life. Even though it is a hard life, there is always something to be positive and happy about

Come along and share the joys, sorrows, triumphs and tribulations of my life. Experience the growth of my faith.....and also my questioning of society's values.